CD8+ Lymphomatoid Papulosis and Its Differential Diagnosis

Abstract

We describe 5 cases (4 males, 14-43 years old; 1 female, 61 years old) of primary cutaneous T-cell lymphoproliferative lesions expressing a CD8/granzyme/CD30-positive phenotype. Four cases were compatible with lymphomatoid papulosis (LyP) based on the clinical course, which was recurrent asymptomatic papular nodular lesions over years responding to methotrexate; granulomatous inflammation and lack of other inflammatory cell elements were characteristic. In 1 case, an initial erroneous diagnosis was made of aggressive epidermotropic CD8+ T-cell lymphoma. The fifth case in this series was first interpreted as representing primary cutaneous anaplastic large cell lymphoma but was later recategorized as primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma owing to the extent of extracutaneous dissemination, including testicular involvement and disease progression despite chemotherapeutic intervention. Although all cases of LyP showed sharp cytoplasmic membrane staining with perinuclear Golgi accentuation with CD30, the recategorized case of aggressive epidermotropic CD8 cytotoxic T-cell lymphoma manifested only a weak cytoplasmic staining pattern. CD8 LyP defines a distinctive entity with characteristic light microscopic and phenotypic findings and has a predilection for young males. CD30 expression can occur in other forms of CD8 lymphoproliferative disease unrelated to primary cutaneous anaplastic large cell lymphoma or LyP.

CD8+ Lymphomatoid Papulosis

“…Therefore, further study will be needed to establish the cardinal characteristic histopathologic findings of CD8＋ LyP. The differential diagnosis of CD8＋ LyP includes primary cutaneous aggressive epidermotropic CD8＋ T-cell lymphoma (CTCL) and pagetoid reticulosis 2,5,7 . The cornerstone for distinguishing between these disease entities is the clinicopathologic correlation.…”

Type D Lymphomatoid Papulosis: An uncommon Variant. A case report and review of the literature

“…However, the classification system for cutaneous lymphomas has evolved rapidly, and, during consensus meetings in 2003-2004, the World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) classification grouped lymphomatoid papulosis among the indolent cutaneous T-cell lymphomas within the spectrum of CD30-positive lymphoproliferative disorders. The rationale for classifying lymphomatoid papulosis as a cutaneous lymphoma is its association with other malignant lymphoproliferative disorders; however, even today some experts hesitate to classify this chronic skin disease as a true malignancy because of its spontaneous resolution and benign clinical course considering it as a pseudolymphomatous inflammatory process [1,7,8]. It was not until recently that there were just three histological variants of LyP known (A, B and C).…”

“…Histopathological findings. The tipical lesions of LyP present as a wedge infiltrate with a varied number of large atypical cells that can be solitary or clustered [1,11]. Since 2010, there are 4 histological types of LyP well described [2,4,11] Inmunohistochemical findings.…”

“…Unlike LyP, the infiltrate tends to be more superficial and not wedge shaped, and tumor cells tend to be almost exclusively confined to the epidermis. Interestingly, CD30 positivity has also been increasingly reported in PR, and so confident distinction from this variant of LyP based on the immunophenotype alone is not reliable and ultimately depends on clinicopathologic correlation [1][2][3][4]. Clinical Course and Prognosis.…”

“…Nevertheless, LyP is recognized as a neoplastic process lying at the benign end of the spectrum of CD30+ lymphoproliferative diseases [1]. Clinically it is defined as a rhythmic paradoxical chronic, recurrent, self-healing eruption of erythematous papules and small nodules, characterized by a waxing and waning course and by histopathologic features of cutaneous T-cell lymphoma [2,3].…”